Overview

  • Product name

  • Description

    Rabbit polyclonal to NSDHL
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, ICC/IFmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow
  • Immunogen

    Recombinant fragment corresponding to Human NSDHL aa 154-211.
    Sequence:

    DIKNGTEDLPYAMKPIDYYTETKILQERAVLGANDPEKNFLTTAIRPHGI FGPRDPQL


    Database link: Q15738

  • Positive control

    • IHC-P: Human liver and cervical cancer tissues. ICC/IF: HeLa cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab237799 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
IHC-P 1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
ICC/IF 1/50 - 1/200.

Target

  • Tissue specificity

    Brain, heart, liver, lung, kidney, skin and placenta.
  • Pathway

    Steroid biosynthesis; zymosterol biosynthesis; zymosterol from lanosterol: step 4/6.
  • Involvement in disease

    Defects in NSDHL are the cause of congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD) [MIM:308050]. CHILD is an X-linked dominant disorder of lipid metabolism with disturbed cholesterol biosynthesis, which typically results in male lethality. Clinically, it is characterized by congenital, unilateral, ichthyosisform erythroderma with striking lateralization, sharp midline demarcation, and ipsilateral limb defects and hypoplasia of the body. Limbs defects range from hypoplasia of digits or ribs to complete amelia, often including scoliosis.
    Defects in NSDHL are the cause of CK syndrome (CKS) [MIM:300831]. CKS is a disorder characterized by mild to severe cognitive impairment, seizures, microcephaly, cerebral cortical malformations, dysmorphic facial features, and thin body habitus.
  • Sequence similarities

    Belongs to the 3-beta-HSD family.
  • Cellular localization

    Membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • decarboxylating antibody
    • H105E3 antibody
    • H105e3 protein antibody
    • NAD(P) dependent steroid dehydrogenase like antibody
    • NSDHL antibody
    • NSDHL_HUMAN antibody
    • Protein H105e3 antibody
    • SDR31E1 antibody
    • Short chain dehydrogenase/reductase family 31E member 1 antibody
    • Sterol 4 alpha carboxylate 3 dehydrogenase decarboxylating antibody
    • Sterol-4-alpha-carboxylate 3-dehydrogenase antibody
    • XAP104 antibody
    see all

Images

  • Paraffin-embedded human liver tissue stained for NSDHL using ab237799 at 1/400 dilution in immunohistochemical analysis.

    After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

  • Paraffin-embedded human cervical cancer tissue stained for NSDHL using ab237799 at 1/400 dilution in immunohistochemical analysis.

    After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

  • 4% Formaldehyde-fixed, 0.2% Triton X-100 permeabilized HeLa (human epithelial cell line from cervix adenocarcinoma) cells stained for NSDHL (green) using ab237799 at 1/133 dilution in ICC/IF, followed by Alexa Fluor 488® conjugated Goat Anti-Rabbit IgG (H+L). Counter-stained with DAPI.

References

ab237799 has not yet been referenced specifically in any publications.

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